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Epidural labour analgesia in a parturient with Noonan syndrome: a case report

[Analgésie péridurale chez une parturiente atteinte du syndrome de Noonan : une présentation de cas]

Joelle McBain, BSc, MD^*, Edmond G. Lemire, MD PhD FRCPC FCCMG and David C. Campbell, MD MSc FRCPC^*

^* From the Departments of Anesthesia, and
Pediatrics, College of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.

Address correspondence to: Dr. David C. Campbell, Department of Anesthesia, College of Medicine, University of Saskatchewan, Royal University Hospital, 103 Hospital Drive, Saskatoon, Saskatchewan S7N 0W8, Canada. Phone: 306-655-1183; Fax: 306-655-1279; E-mail: david.campbell{at}saskatoonhealthregion.ca

Purpose: Noonan syndrome is a relatively uncommon genetic disorder with implications for anesthesia due to multiple organ system involvement. Pregnancy presents additional concerns and there are only four reported cases of anesthesia for Cesarean delivery in parturients with Noonan syndrome. We describe the first reported management of labour analgesia in a parturient with Noonan syndrome culminating in vaginal delivery.

Clinical features: A 21-yr-old parturient with Noonan syndrome received patient-controlled epidural analgesia for labour at 39 weeks gestation. Meticulous attention to the anesthetic technique resulted in good analgesia, and a successful outcome for mother and child. The different approaches to labour analgesia in parturients, with particular attention to combined spinal epidural vs epidural analgesia in this setting are discussed.

Conclusion: Parturients with Noonan syndrome can present with an array of anomalies that may present difficulties to the anesthesiologist including a difficult airway, cardiopulmonary abnormalities, exaggerated lumbar lordosis and short stature. Careful preoperative consultation and determination of the degree of associated anomalies will help to prepare the anesthesiologist for potential problems.